A 73-year-old Japanese male, who had a history of alcoholic cirrhosis, was admitted to Saga University Hospital to receive treatment for hepatocellular carcinoma. The patient was treated to maintain his liver function, however, the total bilirubin level continued to increase gradually. Endoscopic retrograde cholangiography demonstrated an obstruction of the bilateral intrahepatic ducts. Although endoscopic nasobiliary drainage was performed, the patient died two months after admission. At the autopsy, multiple peribiliary cysts were found to almost completely obstruct the bilateral intrahepatic bile ducts. In addition, hepatolithiasis was found in the right hepatic duct. To date, only a few such cases of multipleperibiliary cysts with obstructive jaundice have been reported, and no such case accompanied by hepatolithiasis. It is also important to note that all the reported cases resulted in a poor prognosis. We herein report a very rare autopsy case with obstructive jaundice due to multiple peribiliary cysts accompanying hepatolithiasis. Generally, multiple peribiliary cysts are considered to be clinically harmless, but once they cause obstructive jaundice, their presence suggests the occurrence of end-stage cirrhosis and a poor prognosis.